The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis

A metabolic switch toward lipid use in glycolytic muscle is an early pathologic event in a mouse model of amyotrophic lateral sclerosis

(2015) L. Palamiuc et al.   EMBO Molecular Medicine

Tweak regulates astrogliosis, microgliosis and skeletal muscle atrophy in a mouse model of amyotrophic lateral sclerosis

(2015) M. Bowerman et al.   HUMAN MOLECULAR GENETICS

ALS-Linked P56S-VAPB Mutation Impairs the Formation of Multinuclear Myotube in C2C12 Cells

(2015) Yukako Tokutake et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Glycoprotein nonmetastatic melanoma protein B ameliorates skeletal muscle lesions in a SOD1G93Amouse model of amyotrophic lateral sclerosis

(2015) Yuki Nagahara et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Early ALS-type gait abnormalities in AMP-dependent protein kinase-deficient mice suggest a role for this metabolic sensor in early stages of the disease

(2015) Maxime Vergouts et al.   METABOLIC BRAIN DISEASE

Activation of the endoplasmic reticulum stress response in skeletal muscle of G93A*SOD1 amyotrophic lateral sclerosis mice

(2015) Dapeng Chen et al.   Frontiers in Cellular Neuroscience

VCP-dependent muscle degeneration is linked to defects in a dynamic tubular lysosomal network in vivo

(2015) Alyssa E Johnson et al.   eLife

Motoneuronal and muscle-selective removal of ALS-related misfolded proteins: Figure 1

(2015) Valeria Crippa et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Time-Point Dependent Activation of Autophagy and the UPS in SOD1G93A Mice Skeletal Muscle

(2015) Sara Oliván et al.   PLoS One

Perturbations in intracellular Ca2+ handling in skeletal muscle in the G93A*SOD1 mouse model of amyotrophic lateral sclerosis

(2014) Eva R. Chin et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Neuronal cells but not muscle cells are resistant to oxidative stress mediated protein misfolding and cell death: Role of molecular chaperones

(2014) Arunabh Bhattacharya et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Early Gene Expression Changes in Skeletal Muscle from SOD1G93A Amyotrophic Lateral Sclerosis Animal Model

(2014) Gabriela P. de Oliveira et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Changes in skeletal muscle iron metabolism outpace amyotrophic lateral sclerosis onset in transgenic rats bearing the G93A hmSOD1 gene mutation

(2014) M. Halon et al.   FREE RADICAL RESEARCH

Extra virgin olive oil intake delays the development of amyotrophic lateral sclerosis associated with reduced reticulum stress and autophagy in muscle of SOD1G93A mice

(2014) Sara Oliván et al.   JOURNAL OF NUTRITIONAL BIOCHEMISTRY

Skeletal muscle oxidative capacity in amyotrophic lateral sclerosis

(2014) Terence E. Ryan et al.   MUSCLE & NERVE

Muscle Expression of Mutant Androgen Receptor Accounts for Systemic and Motor Neuron Disease Phenotypes in Spinal and Bulbar Muscular Atrophy

(2014) Constanza J. Cortes et al.   NEURON

MicroRNA-206: A Potential Circulating Biomarker Candidate for Amyotrophic Lateral Sclerosis

(2014) Janne M. Toivonen et al.   PLoS One

The Role of Muscle microRNAs in Repairing the Neuromuscular Junction

(2014) Gregorio Valdez et al.   PLoS One

Fast Skeletal Muscle Troponin Activator tirasemtiv Increases Muscle Function and Performance in the B6SJL-SOD1G93A ALS Mouse Model

(2014) Darren T. Hwee et al.   PLoS One

Skeletal Muscle Satellite Cells in Amyotrophic Lateral Sclerosis

(2014) Annarita Scaramozza et al.   ULTRASTRUCTURAL PATHOLOGY

ALS as a distal axonopathy: molecular mechanisms affecting neuromuscular junction stability in the presymptomatic stages of the disease

(2014) Elizabeth B. Moloney et al.   Frontiers in Neuroscience

Mitochondrial abnormalities and low grade inflammation are present in the skeletal muscle of a minority of patients with amyotrophic lateral sclerosis; an observational myopathology study

(2014) Safa Al-Sarraj et al.   Acta Neuropathologica Communications

Protein aggregation in amyotrophic lateral sclerosis

(2013) Anna M. Blokhuis et al.   ACTA NEUROPATHOLOGICA

Differential effects of mutant SOD1 on protein structure of skeletal muscle and spinal cord of familial amyotrophic lateral sclerosis: Role of chaperone network

(2013) Rochelle Wei et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Muscle histone deacetylase 4 upregulation in amyotrophic lateral sclerosis: potential role in reinnervation ability and disease progression

(2013) Gaëlle Bruneteau et al.   BRAIN

Axonal degeneration in the peripheral nervous system: Implications for the pathogenesis of amyotrophic lateral sclerosis

(2013) Lindsey R. Fischer-Hayes et al.   EXPERIMENTAL NEUROLOGY

Synergistic Effects of GDNF and VEGF on Lifespan and Disease Progression in a Familial ALS Rat Model

(2013) Dan Krakora et al.   MOLECULAR THERAPY

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Spinal inhibitory circuits and their role in motor neuron degeneration

(2013) Uri Nimrod Ramírez-Jarquín et al.   NEUROPHARMACOLOGY

Absence of Nrf2 or Its Selective Overexpression in Neurons and Muscle Does Not Affect Survival in ALS-Linked Mutant hSOD1 Mouse Models

(2013) Marcelo R. Vargas et al.   PLoS One

Systemic Down-Regulation of Delta-9 Desaturase Promotes Muscle Oxidative Metabolism and Accelerates Muscle Function Recovery following Nerve Injury

(2013) Ghulam Hussain et al.   PLoS One

Early Changes of Neuromuscular Transmission in the SOD1(G93A) Mice Model of ALS Start Long before Motor Symptoms Onset

(2013) Mariana C. Rocha et al.   PLoS One

TDP-43, an ALS Linked Protein, Regulates Fat Deposition and Glucose Homeostasis

(2013) Nancy R. Stallings et al.   PLoS One

Defective Mitochondrial Dynamics Is an Early Event in Skeletal Muscle of an Amyotrophic Lateral Sclerosis Mouse Model

(2013) Guo Luo et al.   PLoS One

Bone Marrow Transplantation in Hindlimb Muscles of Motoneuron Degenerative Mice Reduces Neuronal Death and Improves Motor Function

(2013) Diego Pastor et al.   STEM CELLS AND DEVELOPMENT

Differential autophagy power in the spinal cord and muscle of transgenic ALS mice

(2013) Valeria Crippa et al.   Frontiers in Cellular Neuroscience

Postnatal muscle modification by myogenic factors modulates neuropathology and survival in an ALS mouse model

(2013) Kevin H. J. Park et al.   Nature Communications

VAPB/ALS8 MSP Ligands Regulate Striated Muscle Energy Metabolism Critical for Adult Survival in Caenorhabditis elegans

(2013) Sung Min Han et al.   PLoS Genetics

Safety, tolerability and pharmacodynamics of a skeletal muscle activator in amyotrophic lateral sclerosis

(2012) Jeremy Shefner et al.   Amyotrophic Lateral Sclerosis

The molecular basis of the frontotemporal lobar degeneration–amyotrophic lateral sclerosis spectrum

(2012) Tim van Langenhove et al.   ANNALS OF MEDICINE

Molecular Signatures of Amyotrophic Lateral Sclerosis Disease Progression in Hind and Forelimb Muscles of an SOD1G93A Mouse Model

(2012) Daniele Capitanio et al.   ANTIOXIDANTS & REDOX SIGNALING

Protein misfolding, mitochondrial dysfunction and muscle loss are not directly dependent on soluble and aggregation state of mSOD1 protein in skeletal muscle of ALS

(2012) Rochelle Wei et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity

(2012) Christel Dentel et al.   BRAIN

Elevated PGC-1α Activity Sustains Mitochondrial Biogenesis and Muscle Function without Extending Survival in a Mouse Model of Inherited ALS

(2012) Sandrine Da Cruz et al.   Cell Metabolism

Amyotrophic Lateral Sclerosis and Excitotoxicity: From Pathological Mechanism to Therapeutic Target

(2012) E. Bogaert et al.   CNS & Neurological Disorders-Drug Targets

Decreased mRNA Expression of PGC-1α and PGC-1α-Regulated Factors in the SOD1G93AALS Mouse Model and in Human Sporadic ALS

(2012) Nadine Thau et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Analysis of neuromuscular junctions and effects of anabolic steroid administration in the SOD1G93A mouse model of ALS

(2012) Valentina Cappello et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Alterations of signaling pathways in muscle tissues of patients with amyotrophic lateral sclerosis

(2012) Fei Yin et al.   MUSCLE & NERVE

Disruption of skeletal muscle mitochondrial network genes and miRNAs in amyotrophic lateral sclerosis

(2012) Aaron P. Russell et al.   NEUROBIOLOGY OF DISEASE

Altered in vitro Proliferation of Mouse SOD1-G93A Skeletal Muscle Satellite Cells

(2012) Raquel Manzano et al.   Neurodegenerative Diseases

Dihydrotestosterone Ameliorates Degeneration in Muscle, Axons and Motoneurons and Improves Motor Function in Amyotrophic Lateral Sclerosis Model Mice

(2012) Young-Eun Yoo et al.   PLoS One

Mitochondrial genome aberrations in skeletal muscle of patients with motor neuron disease

(2012) Lucia Artuso et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Intramuscular administration of a VEGF zinc finger transcription factor activator (VEGF-ZFP-TF) improves functional outcomes in SOD1 rats

(2011) Michele A. Kliem et al.   Amyotrophic Lateral Sclerosis

Abnormalities of satellite cells function in amyotrophic lateral sclerosis

(2011) Pierre-François Pradat et al.   Amyotrophic Lateral Sclerosis

Proteomic analysis of muscle affected by motor neuron degeneration: The wobbler mouse model of amyotrophic lateral sclerosis

(2011) Lisa Staunton et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Hind limb muscle atrophy precedes cerebral neuronal degeneration in G93A-SOD1 mouse model of amyotrophic lateral sclerosis: A longitudinal MRI study

(2011) Stefania Marcuzzo et al.   EXPERIMENTAL NEUROLOGY

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease

(2011) Thomas Philips et al.   LANCET NEUROLOGY

Impaired expression of insulin-like growth factor-1 system in skeletal muscle of amyotrophic lateral sclerosis patients

(2011) Christian Lunetta et al.   MUSCLE & NERVE

Altered Expression of Myogenic Regulatory Factors in the Mouse Model of Amyotrophic Lateral Sclerosis

(2011) Raquel Manzano et al.   Neurodegenerative Diseases

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

The anabolic/androgenic steroid nandrolone exacerbates gene expression modifications induced by mutant SOD1 in muscles of mice models of amyotrophic lateral sclerosis

(2011) Mariarita Galbiati et al.   PHARMACOLOGICAL RESEARCH

Physiological characterization of human muscle acetylcholine receptors from ALS patients

(2011) E. Palma et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial dysfunction in ALS

(2011) Mauro Cozzolino et al.   PROGRESS IN NEUROBIOLOGY

Muscle atrophy induced by SOD1G93A expression does not involve the activation of caspase in the absence of denervation

(2011) Gabriella Dobrowolny et al.   Skeletal Muscle

Investigation of MGF mRNA expression in patients with amyotrophic lateral sclerosis using parallel in vivo and in vitro approaches

(2010) Richard M. Evans et al.   Amyotrophic Lateral Sclerosis

Mitochondrial Respiratory Chain Dysfunction in Muscle From Patients With Amyotrophic Lateral Sclerosis

(2010) Veronica Crugnola et al.   ARCHIVES OF NEUROLOGY

Oxidative stress in skeletal muscle stimulates early expression of Rad in a mouse model of amyotrophic lateral sclerosis

(2010) Benoît Halter et al.   FREE RADICAL BIOLOGY AND MEDICINE

Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice

(2010) Margaret Wong et al.   HUMAN MOLECULAR GENETICS

Neuronal NOS is dislocated during muscle atrophy in amyotrophic lateral sclerosis

(2010) Naoki Suzuki et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Astrogliosis in amyotrophic lateral sclerosis: Role and therapeutic potential of astrocytes

(2010) Marcelo R. Vargas et al.   Neurotherapeutics

A soluble activin type IIB receptor improves function in a mouse model of amyotrophic lateral sclerosis

(2009) Brett M. Morrison et al.   EXPERIMENTAL NEUROLOGY

Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

(2009) Siân C. Barber et al.   FREE RADICAL BIOLOGY AND MEDICINE

Hyperactive Intracellular Calcium Signaling Associated with Localized Mitochondrial Defects in Skeletal Muscle of an Animal Model of Amyotrophic Lateral Sclerosis

(2009) Jingsong Zhou et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Progressive motor unit loss in the G93A mouse model of amyotrophic lateral sclerosis is unaffected by gender

(2009) J. Hegedus et al.   MUSCLE & NERVE

Functional over-load saves motor units in the SOD1-G93A transgenic mouse model of amyotrophic lateral sclerosis

(2009) T. Gordon et al.   NEUROBIOLOGY OF DISEASE

Muscle Mitochondrial Uncoupling Dismantles Neuromuscular Junction and Triggers Distal Degeneration of Motor Neurons

(2009) Luc Dupuis et al.   PLoS One

MicroRNA-206 Delays ALS Progression and Promotes Regeneration of Neuromuscular Synapses in Mice

(2009) A. H. Williams et al.   SCIENCE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Presymptomatic biochemical changes in hindlimb muscle of G93A human Cu/Zn superoxide dismutase 1 transgenic mouse model of amyotrophic lateral sclerosis

(2008) Kevin H.J. Park et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity

(2008) Gabriella Dobrowolny et al.   Cell Metabolism

Mechano-growth factor, an IGF-I splice variant, rescues motoneurons and improves muscle function in SOD1G93A mice

(2008) Joanna Riddoch-Contreras et al.   EXPERIMENTAL NEUROLOGY

The deacetylase HDAC4 controls myocyte enhancing factor-2-dependent structural gene expression in response to neural activity

(2008) Todd J. Cohen et al.   FASEB JOURNAL

Direct Muscle Delivery of GDNF With Human Mesenchymal Stem Cells Improves Motor Neuron Survival and Function in a Rat Model of Familial ALS

(2008) Masatoshi Suzuki et al.   MOLECULAR THERAPY

Local expression of mIgf-1 modulates ubiquitin, caspase and CDK5 expression in skeletal muscle of an ALS mouse model

(2008) Gabriella Dobrowolny et al.   NEUROLOGICAL RESEARCH

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE